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AMYLOIDOSIS
1378
expected, for, by the time one stone has become manifest clinically, so many small concretions may be present that reduction in calcium intake may not reverse the process of further stone formation. As Carr suggests, the only cure may then be excision of the affected lobe of the kidney. Flocks 5 has also found that hypercalcinuria is common in patients with urinary stone, and yet in 21 out of 35 cases there was no evidence of hyperparathyroidism or bone disease. Increased intestinal absorption may therefore be responsible-a fact which emphasises that this problem will not be solved by urologists, radiologists, or physicians alone. AMYLOIDOSIS
WHILE the cause and method of deposition of amyloid in the tissues remain unknown, the vagaries of incidence are becoming clearer. In contrast to those cases in which the amyloid disease is secondary to some known predisposing cause-such as chronic sepsis, syphilis, tuberculosis, tumours, and the more recently recognised myelomatosis, rheumatoid arthritis, and regional enteritis -there are cases of " primary amyloidosis " in which no recognised cause can be found, some of these falling into definite syndromes according to the organs mainly or primarily involved. Amyloid disease affecting the central nervous system sometimes escapes diagnosis. 6 Chambers et a1. report three neurological cases in which men in the early thirties developed a form of polyneuritis or symptoms mimicking tabes. In the nerves there was obvious enlargement, and biopsy revealed amyloid, which was also sometimes found post mortem under the arachnoid, in the spinal dura mater, and throughout the posterior-root ganglia. Chambers et al. found reports of a familial form of primary amyloidosis affecting mainly the nervous system. The heart is commonly affected in primary amyloidosis, especially in older patients; the symptoms and signs are those of progressive myocardial failure, and, when no other cause can be found, amyloid disease should certainly be suspected. The amyloid may be deposited extensively under the endocardium alone or also under the pericardium, or it may infiltrate the myocardium; in some cases reported by Symmers the muscle-fibres were actually transformed into amyloid substance. The tongue is also sometimes involved in primary amyloidosis, causing macroglossia which may lead to difficulty in speaking, eating, and protrusion. As in the heart, the amyloid may be interstitial, extending out even to the gums (biopsy of which may give the diagnosis), or it may involve the muscle-fibres themselves. The skin may be involved in various ways. One of the patients in the series reported by Chambers et al. initially consulted a dermatologist on account of trophic lesions resulting from the polyneuritis; haemorrhagic lesions or generalised pigmentation may develop; or there may be nodular or widespread thickening due to amyloid deposition, as in an otherwise healthy young woman with involvement of the areolae and vulva.3 The kidneys are commonly involved in secondary amyloidosis; and in the primary form hxmaturia, the nephrotic state, or even urxmia may be the only or first sign. The alimentary tract is also liable to be involved in primary amyloid disease, as also in the secondary form. 5. 6.
Flocks, R. H. ibid. 1939, 113, 1466. Cappell, D. F. Muir’s Textbook of Pathology; 6th ed., p. 150. London,
1951. 7. Chambers, R. A., Medd, W. E., Spencer; H. Quart. 8. Symmers, W. St.C. J. clin. Path. 1956, 9, 212.
J. Med. 1958, 27, 207.
There was a tendency 9 to regard primary amyloidosis as a separate entity from the secondary type. Besides the different distribution and the lack of a recognised predisposing cause, the primary form seemed to be characterised by different staining reactions of the amyloid deposits, and in some cases a foreign-body reaction developed around or calcium was deposited in the amyloid. In a comprehensive review Symmers 10 showed that the sites involved in primary cases may also be involved in secondary, though the incidence may be very different; all the other features can occur in either form of the disease, and accordingly all varieties are related to one fundamental disturbance. He suggests that amyloid is an insoluble glycoprotein laid down in the connective-tissue ground-substance as the result of a local antigen-antibody reaction. Cameron 11admits that where it comes from and how it is produced are baffling problems. Both these investigators point out that amyloid is closely related to hyaline degeneration, and in biopsy some specific stain (methyl-violet, Congo-red, iodine) must be positive before the diagnosis of amyloid disease is established. STRESS FRACTURES
STRESS fractures of
leg bones are often overlooked; for uncommonly they are detectable only with special radiographic projections. In the 1939-45 war, fatigue fractures of the tibia were described by Hartley 12 in children and young adults and by Leveton 13 in Army recruits. In the young patient these fractures affect the upper tibia, and often spread from a small crack across the bone to produce a complete fracture. In the middle-aged and elderly, fatigue fractures tend to involve the lower end of the tibia.1-1 Most of these patients do not give a history of unusual muscular activity, and the fractures unite fairly rapidly. In two groups of patients stress fracture seems to be an occupational hazard. Burrows 15 has described a minute not
infraction of the tibial cortex in male ballet dancers. The lesion tends to affect the middle of the bone, and gives rise to pain, swelling, and tenderness over the crest of the tibia. Many different radiographs may be necessary to reveal the crack, and in two of these patients there was sufficient doubt about the diagnosis for the lesion to be explored; the specimens removed at operation showed A similar lesion, very little evidence of bone repair. lower in the has been described tibia, slightly by Devas," who thinks that this explains the painful condition of the found in athletes in training and often known as leg " shin splints ". These patients present with pain, usually at the start of training and subsiding with rest. The pain often gets more severe as training becomes more arduous; and radiographs, which may have to be repeated several times before any abnormality is detected, will show merely a small crack running through the cortex. Stress fractures in the tibia are probably a direct result of muscle pull. Treatment of these minute cracks is often difficult. Immobilisation is necessary only so far as it contributes to the patient’s comfort. In time symptoms usually subside, but the disability from this lesion is more protracted than after a complete fracture of the bone. 9. Lubarsch, O. Virchows Arch. 1929, 271, 867. 10. Symmers, W. St.C. J. clin. Path. 1956, 9, 187. 11. Cameron, G. R. Pathology of the Cell; p. 339. London, 1952. 12. Hartley, J. B. Brit. J. Surg. 1942, 30, 9. 13. Leveton, A. L. Amer. J. Surg. 1946, 71, 222. 14. Singer, M., Maudsley, R. H. J. Bone Jt Surg. 1954, 36B, 647. 15. Burrows, H. J. ibid. 1956, 38B, 83. 16. Devas, M. B. ibid. 1958, 40B, 227.
expected, for, by the time one stone has become manifest clinically, so many small concretions may be present that reduction in calcium intake may not reverse the process of further stone formation. As Carr suggests, the only cure may then be excision of the affected lobe of the kidney. Flocks 5 has also found that hypercalcinuria is common in patients with urinary stone, and yet in 21 out of 35 cases there was no evidence of hyperparathyroidism or bone disease. Increased intestinal absorption may therefore be responsible-a fact which emphasises that this problem will not be solved by urologists, radiologists, or physicians alone. AMYLOIDOSIS
WHILE the cause and method of deposition of amyloid in the tissues remain unknown, the vagaries of incidence are becoming clearer. In contrast to those cases in which the amyloid disease is secondary to some known predisposing cause-such as chronic sepsis, syphilis, tuberculosis, tumours, and the more recently recognised myelomatosis, rheumatoid arthritis, and regional enteritis -there are cases of " primary amyloidosis " in which no recognised cause can be found, some of these falling into definite syndromes according to the organs mainly or primarily involved. Amyloid disease affecting the central nervous system sometimes escapes diagnosis. 6 Chambers et a1. report three neurological cases in which men in the early thirties developed a form of polyneuritis or symptoms mimicking tabes. In the nerves there was obvious enlargement, and biopsy revealed amyloid, which was also sometimes found post mortem under the arachnoid, in the spinal dura mater, and throughout the posterior-root ganglia. Chambers et al. found reports of a familial form of primary amyloidosis affecting mainly the nervous system. The heart is commonly affected in primary amyloidosis, especially in older patients; the symptoms and signs are those of progressive myocardial failure, and, when no other cause can be found, amyloid disease should certainly be suspected. The amyloid may be deposited extensively under the endocardium alone or also under the pericardium, or it may infiltrate the myocardium; in some cases reported by Symmers the muscle-fibres were actually transformed into amyloid substance. The tongue is also sometimes involved in primary amyloidosis, causing macroglossia which may lead to difficulty in speaking, eating, and protrusion. As in the heart, the amyloid may be interstitial, extending out even to the gums (biopsy of which may give the diagnosis), or it may involve the muscle-fibres themselves. The skin may be involved in various ways. One of the patients in the series reported by Chambers et al. initially consulted a dermatologist on account of trophic lesions resulting from the polyneuritis; haemorrhagic lesions or generalised pigmentation may develop; or there may be nodular or widespread thickening due to amyloid deposition, as in an otherwise healthy young woman with involvement of the areolae and vulva.3 The kidneys are commonly involved in secondary amyloidosis; and in the primary form hxmaturia, the nephrotic state, or even urxmia may be the only or first sign. The alimentary tract is also liable to be involved in primary amyloid disease, as also in the secondary form. 5. 6.
Flocks, R. H. ibid. 1939, 113, 1466. Cappell, D. F. Muir’s Textbook of Pathology; 6th ed., p. 150. London,
1951. 7. Chambers, R. A., Medd, W. E., Spencer; H. Quart. 8. Symmers, W. St.C. J. clin. Path. 1956, 9, 212.
J. Med. 1958, 27, 207.
There was a tendency 9 to regard primary amyloidosis as a separate entity from the secondary type. Besides the different distribution and the lack of a recognised predisposing cause, the primary form seemed to be characterised by different staining reactions of the amyloid deposits, and in some cases a foreign-body reaction developed around or calcium was deposited in the amyloid. In a comprehensive review Symmers 10 showed that the sites involved in primary cases may also be involved in secondary, though the incidence may be very different; all the other features can occur in either form of the disease, and accordingly all varieties are related to one fundamental disturbance. He suggests that amyloid is an insoluble glycoprotein laid down in the connective-tissue ground-substance as the result of a local antigen-antibody reaction. Cameron 11admits that where it comes from and how it is produced are baffling problems. Both these investigators point out that amyloid is closely related to hyaline degeneration, and in biopsy some specific stain (methyl-violet, Congo-red, iodine) must be positive before the diagnosis of amyloid disease is established. STRESS FRACTURES
STRESS fractures of
leg bones are often overlooked; for uncommonly they are detectable only with special radiographic projections. In the 1939-45 war, fatigue fractures of the tibia were described by Hartley 12 in children and young adults and by Leveton 13 in Army recruits. In the young patient these fractures affect the upper tibia, and often spread from a small crack across the bone to produce a complete fracture. In the middle-aged and elderly, fatigue fractures tend to involve the lower end of the tibia.1-1 Most of these patients do not give a history of unusual muscular activity, and the fractures unite fairly rapidly. In two groups of patients stress fracture seems to be an occupational hazard. Burrows 15 has described a minute not
infraction of the tibial cortex in male ballet dancers. The lesion tends to affect the middle of the bone, and gives rise to pain, swelling, and tenderness over the crest of the tibia. Many different radiographs may be necessary to reveal the crack, and in two of these patients there was sufficient doubt about the diagnosis for the lesion to be explored; the specimens removed at operation showed A similar lesion, very little evidence of bone repair. lower in the has been described tibia, slightly by Devas," who thinks that this explains the painful condition of the found in athletes in training and often known as leg " shin splints ". These patients present with pain, usually at the start of training and subsiding with rest. The pain often gets more severe as training becomes more arduous; and radiographs, which may have to be repeated several times before any abnormality is detected, will show merely a small crack running through the cortex. Stress fractures in the tibia are probably a direct result of muscle pull. Treatment of these minute cracks is often difficult. Immobilisation is necessary only so far as it contributes to the patient’s comfort. In time symptoms usually subside, but the disability from this lesion is more protracted than after a complete fracture of the bone. 9. Lubarsch, O. Virchows Arch. 1929, 271, 867. 10. Symmers, W. St.C. J. clin. Path. 1956, 9, 187. 11. Cameron, G. R. Pathology of the Cell; p. 339. London, 1952. 12. Hartley, J. B. Brit. J. Surg. 1942, 30, 9. 13. Leveton, A. L. Amer. J. Surg. 1946, 71, 222. 14. Singer, M., Maudsley, R. H. J. Bone Jt Surg. 1954, 36B, 647. 15. Burrows, H. J. ibid. 1956, 38B, 83. 16. Devas, M. B. ibid. 1958, 40B, 227.